NR 328 EXAM 2 STUDY GUIDE
HEMATOLOGIC DISORDERS: 7-10 questions
Identify general nursing strategies associated with altered anemia
Describe the morphology, pathophysiology, clinical manifestations,
therapeutic management and nursing considerations of the following
anemias:
o ***Laboratory norms: 6-12 years of age
Hemoglobin:
11.5-15.5 g/dl
Hematocrit:
35-45%
MCV (mean corpuscular volume)
77-95 fL
Measure of average size of RBC
↓ in microcytic; ↑ in macrocytic
MCHC (mean corpuscular Hgb concentration)
31–37 g/dl
Reflection of Hgb concentration
Refer to color; in hypo-, values are ↓
MCH (mean corpuscular Hgb)
25–33 pg/cell
Reflection of Hgb concentration
Refer to color; in hypo-, values are ↓
o Anemia
Condition where the hemoglobin content of the blood is
insufficient to satisfy bodily needs
Causes:
Blood loss
Accelerated hemolysis
Decreased production
Typical Clinical Manifestations:
Fatigue
Dizziness
Weakness
Pallor
↑ heart rate
↑ breathing rate
o Types:
Iron-deficiency anemia—MOST COMMON IN PEDS
Nutritional anemia
Microcytic-hypochromic—small, pale RBC
Who’s at risk?
o Varied
o Premature babies at ↑ risk b/c they don’t get that
blood rush the mom gives the baby late in that 3 rd
trimester, b/c premies are born early—this is why
premature infants need the iron supplements at 2-3
months, whereas term babies don’t need it until 4-6
months
o Multiples and twins at ↑ because only finite amount
of blood available in that last rush of blood; so not
all the babies get enough iron
, o Breast-fed exclusively babies at ↑ risk—breast milk
doesn’t have same iron content as fortified iron
formula
o Kiddos with chronic blood loss—GI bleeders,
hemophiliacs
o Kiddos with poor dietary intake; whole milk doesn’t
have same iron concentration as iron fortified
formula
Pathophysiology:
o Body stores iron that is used for erythropoiesis
o Iron necessary mineral for erythropoiesis to occur
o Not enough iron to transport to the bone marrow to
make RBCs
o Bone marrow still makes RBCs but they are very
small and have a lower hemoglobin concentration
Clinical Manifestations:
o Typical signs of anemia plus:
Irritability—due to tissue hypoxia/O2 craving
Pica (especially ice, dirt, chalk, powder or
pure starch)—eating of substances that are
not typical of kiddos to eat; however this
could be hard to distinguish from this
occurring normally in toddlerhood
Poor muscle tone
Growth retardation
Headache
Nail bed deformities called?? Spoon nail
Tachycardic; possible murmurs
Lab Values:
o Everything is ↓↓↓↓ b/c its microcytic-hypochromic
Implementations:
o EDUCATION***
Prevention*
High iron foods*
Foods that a toddler would eat; eggs,
breads
Whole milk takes up room in belly!!!
So kiddo may not intake enough iron-
rich food b/c they’re not hungry;
remember whole milk does not have
same iron concentration like fortified
formula so if kiddo is drinking equal
amounts of whole milk as he/she used
to drink the formula he/she is not
getting enough iron
Iron supplements
Best on an empty stomach
Best with Vitamin C
Don’t give with milk
If taking efficiently they will have
black/green tarry stools
Lab values best indicator
Use a straw to prevent staining of
teeth
Monitor for constipation & treat
, Beta-Thallasemia
Hemolytic anemia; bone marrow issue is the underlying
patho—blood cells are extremely unstable, RBCs lyse very
easily
Not producing correct beta chains of hgb—alpha and
gamma attempt to take over and produce RBCs that are
fragile and prone to lysing
***kiddos BM is not making right hemoglobin
chains—they break down very quickly and easily
Microcytic-hypochromic
Defective synthesis of the protein component of
hemoglobin
Who’s at Risk
o Mediterranean descent—Italians and Greeks
Pathophysiology:
o Hemoglobin synthesis is impaired
o Fragile RBC w/short life
o Severe anemia
Types:
o Major
Most severe
Occurs in infancy
If kiddo doesn’t receive freq. transfusions
and treatments they rarely survive childhood
Without treatment they die before age 7
o Intermediate
Develop normally into adulthood
Need to be treated
Delayed puberty
o Minor
Carry the trait, don’t have it
Have normal life-span
Clinical Manifestations:
* occurring b/c of BM involvement
o Frequent epistaxis*
o Osteoporosis*
o Pathologic fractures*
o Chronic CHF
o Myocardia fibrosis
o Murmurs
o Hepatosplenomegaly—b/c spleen has to work
harder to clean up dysfunctional RBCs
o Diabetes mellitus
o Darkening of the skin—indication of iron toxicity
that’s occurring
Therapeutic Management:
o Frequent blood transfusion
Subsequent iron chelation therapy
,
***Enough transfusions to maintain the Hgb
greater than 9.5
Monitor for iron overload
o Splenectomy
Nursing Management:
o Promote compliance
o Assist w/ coping strategies
o Observe for complications of multiple transfusions
o Differentiate between Iron Deficiency Anemia & Thalassemia
IDA Thalassemia
Ferritin Levels
Serum iron
TIBC
MCHC
Hemoglobin
Electrophoresis
Ethnic Background
Sickle-cell anemia
Hemolytic anemia
Autosomal recessive condition where normal hemoglobin
is partially or completely replaced by the sickle-shaped,
abnormal S Hgb
Won’t see manifestations until about 6-12 months after
birth b/c kiddo still had fetal hemoglobin circulating; fetal
hemoglobin does not sickle!
Who’s at Risk?
Pathophysiology:
o Biggest trigger is hypoxia—stress, high altitudes,
cold, not breathing well, excessive exercises, low
body or environment temp, anesthesia—watch
closely in OR, dehydration, infection, acidosis
o Hemoglobin S becomes elongated and rigid
o Cells clump together and obstruct capillary blood
flow
o Ischemia and tissue infarction occur beyond the
obstruction
o Hypoxia continues which leads to tissue infarcts—
ischemia of tissue is extremely painful
Crisis experienced:
o Vaso-occlusive crisis
Norm/MC—typically non-life threatening;
associated with a lot of pain
Seriousness depends upon where occlusion
occurs; ex: brain vs. hand
Manifestations
Severe pain #1
Tissue engorgement
Fever
o Sequestration crisis
Life-threatening
Blood pooling within spleen
HEMATOLOGIC DISORDERS: 7-10 questions
Identify general nursing strategies associated with altered anemia
Describe the morphology, pathophysiology, clinical manifestations,
therapeutic management and nursing considerations of the following
anemias:
o ***Laboratory norms: 6-12 years of age
Hemoglobin:
11.5-15.5 g/dl
Hematocrit:
35-45%
MCV (mean corpuscular volume)
77-95 fL
Measure of average size of RBC
↓ in microcytic; ↑ in macrocytic
MCHC (mean corpuscular Hgb concentration)
31–37 g/dl
Reflection of Hgb concentration
Refer to color; in hypo-, values are ↓
MCH (mean corpuscular Hgb)
25–33 pg/cell
Reflection of Hgb concentration
Refer to color; in hypo-, values are ↓
o Anemia
Condition where the hemoglobin content of the blood is
insufficient to satisfy bodily needs
Causes:
Blood loss
Accelerated hemolysis
Decreased production
Typical Clinical Manifestations:
Fatigue
Dizziness
Weakness
Pallor
↑ heart rate
↑ breathing rate
o Types:
Iron-deficiency anemia—MOST COMMON IN PEDS
Nutritional anemia
Microcytic-hypochromic—small, pale RBC
Who’s at risk?
o Varied
o Premature babies at ↑ risk b/c they don’t get that
blood rush the mom gives the baby late in that 3 rd
trimester, b/c premies are born early—this is why
premature infants need the iron supplements at 2-3
months, whereas term babies don’t need it until 4-6
months
o Multiples and twins at ↑ because only finite amount
of blood available in that last rush of blood; so not
all the babies get enough iron
, o Breast-fed exclusively babies at ↑ risk—breast milk
doesn’t have same iron content as fortified iron
formula
o Kiddos with chronic blood loss—GI bleeders,
hemophiliacs
o Kiddos with poor dietary intake; whole milk doesn’t
have same iron concentration as iron fortified
formula
Pathophysiology:
o Body stores iron that is used for erythropoiesis
o Iron necessary mineral for erythropoiesis to occur
o Not enough iron to transport to the bone marrow to
make RBCs
o Bone marrow still makes RBCs but they are very
small and have a lower hemoglobin concentration
Clinical Manifestations:
o Typical signs of anemia plus:
Irritability—due to tissue hypoxia/O2 craving
Pica (especially ice, dirt, chalk, powder or
pure starch)—eating of substances that are
not typical of kiddos to eat; however this
could be hard to distinguish from this
occurring normally in toddlerhood
Poor muscle tone
Growth retardation
Headache
Nail bed deformities called?? Spoon nail
Tachycardic; possible murmurs
Lab Values:
o Everything is ↓↓↓↓ b/c its microcytic-hypochromic
Implementations:
o EDUCATION***
Prevention*
High iron foods*
Foods that a toddler would eat; eggs,
breads
Whole milk takes up room in belly!!!
So kiddo may not intake enough iron-
rich food b/c they’re not hungry;
remember whole milk does not have
same iron concentration like fortified
formula so if kiddo is drinking equal
amounts of whole milk as he/she used
to drink the formula he/she is not
getting enough iron
Iron supplements
Best on an empty stomach
Best with Vitamin C
Don’t give with milk
If taking efficiently they will have
black/green tarry stools
Lab values best indicator
Use a straw to prevent staining of
teeth
Monitor for constipation & treat
, Beta-Thallasemia
Hemolytic anemia; bone marrow issue is the underlying
patho—blood cells are extremely unstable, RBCs lyse very
easily
Not producing correct beta chains of hgb—alpha and
gamma attempt to take over and produce RBCs that are
fragile and prone to lysing
***kiddos BM is not making right hemoglobin
chains—they break down very quickly and easily
Microcytic-hypochromic
Defective synthesis of the protein component of
hemoglobin
Who’s at Risk
o Mediterranean descent—Italians and Greeks
Pathophysiology:
o Hemoglobin synthesis is impaired
o Fragile RBC w/short life
o Severe anemia
Types:
o Major
Most severe
Occurs in infancy
If kiddo doesn’t receive freq. transfusions
and treatments they rarely survive childhood
Without treatment they die before age 7
o Intermediate
Develop normally into adulthood
Need to be treated
Delayed puberty
o Minor
Carry the trait, don’t have it
Have normal life-span
Clinical Manifestations:
* occurring b/c of BM involvement
o Frequent epistaxis*
o Osteoporosis*
o Pathologic fractures*
o Chronic CHF
o Myocardia fibrosis
o Murmurs
o Hepatosplenomegaly—b/c spleen has to work
harder to clean up dysfunctional RBCs
o Diabetes mellitus
o Darkening of the skin—indication of iron toxicity
that’s occurring
Therapeutic Management:
o Frequent blood transfusion
Subsequent iron chelation therapy
,
***Enough transfusions to maintain the Hgb
greater than 9.5
Monitor for iron overload
o Splenectomy
Nursing Management:
o Promote compliance
o Assist w/ coping strategies
o Observe for complications of multiple transfusions
o Differentiate between Iron Deficiency Anemia & Thalassemia
IDA Thalassemia
Ferritin Levels
Serum iron
TIBC
MCHC
Hemoglobin
Electrophoresis
Ethnic Background
Sickle-cell anemia
Hemolytic anemia
Autosomal recessive condition where normal hemoglobin
is partially or completely replaced by the sickle-shaped,
abnormal S Hgb
Won’t see manifestations until about 6-12 months after
birth b/c kiddo still had fetal hemoglobin circulating; fetal
hemoglobin does not sickle!
Who’s at Risk?
Pathophysiology:
o Biggest trigger is hypoxia—stress, high altitudes,
cold, not breathing well, excessive exercises, low
body or environment temp, anesthesia—watch
closely in OR, dehydration, infection, acidosis
o Hemoglobin S becomes elongated and rigid
o Cells clump together and obstruct capillary blood
flow
o Ischemia and tissue infarction occur beyond the
obstruction
o Hypoxia continues which leads to tissue infarcts—
ischemia of tissue is extremely painful
Crisis experienced:
o Vaso-occlusive crisis
Norm/MC—typically non-life threatening;
associated with a lot of pain
Seriousness depends upon where occlusion
occurs; ex: brain vs. hand
Manifestations
Severe pain #1
Tissue engorgement
Fever
o Sequestration crisis
Life-threatening
Blood pooling within spleen
